Therapeutic modalities in thrombotic thrombocytopenic purpura management among Jehovah’s Witness patients: A review of reported cases

Abstract

IntroductionDevout members of the Jehovah’s Witness faith flatly refuse transfusions of white blood cells, red blood cells, platelets, and plasma. The latter agent is a mainstay in the treatment of thrombotic thrombocytopenic purpura (TTP). Alternative treatment options for Jehovah’s Witness patients are needed and reviewed herein. MethodsCases of TTP treatment among Jehovah’s Witnesses were obtained from the published literature. Key baseline and clinical data were extracted and summarized. ResultsA total of 13 reports spanning a 23-year period and 15 TTP episodes were identified. Median (IQR) age was 45.5 (29.0–57.5) and 12/13 (93%) patients were female. Neurologic symptoms were present in 7/15 (47%) episodes at presentation. Disease confirmation with ADAMTS13 testing was present in 11/15 (73%) of episodes. Corticosteroids and rituximab were employed in 13/15 (87%) and 12/15 (80%) of cases, respectively, with apheresis-based therapy employed in 9/15 (60%) episodes. For eligible cases, caplacizumab was used in 4/5 (80%) episodes; average time to platelet response was shortest in these cases. Sources of exogenous ADAMTS13 accepted by patients in this series included cryo-poor plasma, FVIII concentrate, and cryoprecipitate. Conclusions: Successful management of TTP within the boundaries of the Jehovah’s Witness faith is possible.